CASE REPORT

Subcutaneous Implantable Cardioverter Defibrillator

Lead Failure due to Twiddler Syndrome
KIRSTEN M. KOOIMAN, P.A., TOM F. BROUWER, M.D., VOKKO P. VAN HALM, M.D.,
and REINOUD E. KNOPS, M.D.
From the Department of Clinical and Experimental Cardiology, Heart Center, Amsterdam Medical Center, University
of Amsterdam, Amsterdam, the Netherlands

We present a case of Twiddler syndrome in a patient with a subcutaneous implantable cardioverter

defibrillator (S-ICD). The patient presented herself to the outpatient clinic with pain in the left chest.
Chest x-ray confirmed Twiddler syndrome and ICD read-out revealed lead failure resulting in absent
heart rhythm sensing in one vector. The lead and pulse generator were extracted and a new S-ICD system
was reimplanted submuscular underneath the serratus anterior muscle to prevent reoccurrence. Lead
investigation revealed an insulation defect caused by excessive mechanical stress. (PACE 2015; 38:1369
1371)
defibrillation ICD, sensing, complication, Twiddler
Background
Twiddler syndrome has previously been described in abdominal and transvenous pacemakers
and implantable cardioverter defibrillators (ICDs).
It is caused by rotation of the pulse generator
resulting in coiling of the lead, and most often seen
in obese patients and patients with too large pulse
generator pockets.1 The clinical manifestation of
Twiddler syndrome often involves malfunction
of the device and may result in unnecessary
pacing, inability to shock ventricular arrhythmias,
inappropriate shocks, and mechanical injury of
the heart and vasculature.2 We present the first
reported case of Twiddler syndrome in a patient
with a subcutaneous-ICD (S-ICD) and lead failure
of this relative new system.
Patient Presentation
A 40-year-old woman known with a transposition of the great arteries for which she underwent a
Senning procedure, a concomitant resynchronization pacemaker, and an S-ICD due to obstruction
of the artificial baffle for primary prevention.
She presented herself to our outpatient clinic

Disclosures: Kirsten M. Kooiman, Tom F. Brouwer, and Vokko

P. van Halm have nothing to disclose. Reinoud E. Knops has
received a research grant and a consulting fee from Boston
Scientific, manufacturer of the subcutaneous ICD.
Address for reprints: Kirsten M. Kooiman, P.A., Department of
Cardiology, Academic Medical Center, Meibergdreef 9, 1105
AZ, Amsterdam, the Netherlands. Fax: 3120-5666809; e-mail:
k.m.kooiman@amc.uva.nl
Received August 7, 2015; revised August 19, 2015; accepted
August 19, 2015.
doi: 10.1111/pace.12741

with severe pain underneath the left breast and

at the xiphoid. Read-out of the S-ICD showed
no detectable QRS complexes in the alternate
sense vector. The primary and the secondary
sensing vectors showed morphologically identical
QRS complexes that had changed significantly
since implant (Fig. 1). Impedance of the shock
electrode was within range, and there were no
stored treated or untreated episodes. A chest x-ray
was performed, which revealed medial migration
of the pulse generator and coiling of the lead
(Fig. 2). The patient was admitted, the therapy
of the S-ICD was turned off, and extraction was
scheduled.
S-ICD Extraction and Reimplantation
During extraction of the S-ICD system, a
heavily coiled lead was found in the pocket
(Fig. 3). The pocket was enlarged, but the suture
sleeve at the xiphoid was still intact and prevented
migration of the distal part of the lead toward the
pocket. A new subcutaneous lead was placed in
the original parasternal position, and a new pulse
generator (EMBLEM, Boston Scientific, Marlborough, MA, USA) was positioned underneath the
serratus anterior muscle and fixated with two
nonresorbable sutures. During a follow-up period
of 3 months, there have been no changes in
device position, and there was normal sensing
in all vectors. Repeated chest x-rays showed an
unaltered device position.
Lead Measurements
Postprocedural measurements of the extracted lead were performed in saline and showed
similar impedance values for the primary (1,267
ohms), secondary (1,361 ohms), and shock vector

2015 Wiley Periodicals, Inc.

PACE, Vol. 38

November 2015

1369

KOOIMAN, ET AL.

Figure 1. Captured electrocardiograms of all three sensing vectors directly postimplant and at presentation with
Twiddler syndrome.

Figure 2. Chest x-rays. Panel (A) Directly after initial implant. Panel (B) Presentation with Twiddler syndrome. Panel
(C) After extraction of initial system and reimplantation of new subcutaneous implantable cardioverter defibrillator
system (emblem) under serratus anterior muscle.

(1,277 ohms, coil to can). The impedance for the

alternate vector was <200 ohms. This indicates
that there was an internal insulation defect,
connecting all three electrodes within the lead
(the proximal, distal, and shock electrodes). This
defect explains the absence of the cardiac signal
in the alternate vector and the similarity in
morphology of the QRS complexes in the primary
and secondary vectors, as the proximal and distal
electrodes have become one.

Figure 3. Coiled lead in the pocket during the

extraction procedure.

1370

Discussion
To our knowledge, this report is the first
reported case of an S-ICD lead failure caused
by mechanical stress. Long-term follow-up in
two large registries showed no lead fractures up
to 3 years.3 However, the robust design of the
subcutaneous lead is not immune to excessive
mechanical force. In the current case, the system
was still able to adequately sense the heart rhythm
as the signals could still be obtained in the programmed sensing vector. However, if the device

November 2015

PACE, Vol. 38

S-ICD AND TWIDDLER SYNDROME

would have been programmed in the alternate

sensing vector, this could have resulted in an
inappropriate shock and failure to detect ventricular fibrillation. Changes in QRS morphology,
oversensing, or loss of sensed signals should raise
the suspicion of lead failure. It is important to
realize that the presentation of lead failure in SICDs is different from that in conventional ICD
systems.
In this patient, the pulse generator was
reimplanted underneath the serratus anterior
muscle. The submuscular implantation technique
used in this case is neither necessary nor advisable
for all patients implanted with an S-ICD, as it

is more invasive and causes more postoperative

pain. Particular care should be taken not to damage
the long thoracic nerve, which travels down over
the anterior side of the serratus anterior muscle
and is critical for shoulder function.
Conclusion
We believe this is the first case report of a
Twiddler syndrome in a patient with an S-ICD
with subsequent lead failure. The patient was
implanted with a new lead in the same position
and the pulse generator underneath the serratus
anterior muscle to prevent reoccurrence.

References
1. Boyle NG, Anselme F, Monahan KM, Beswick P, Schuger
CD, Zebede J, Josephson ME. Twiddlers syndrome variants
in ICD patients. Pacing Clin Electrophysiol 1998; 21:2685
2687.

PACE, Vol. 38

2. Chaara J, Sunthorn H. Twiddler syndrome. J Cardiovasc Electrophysiol 2014; 25:659.

3. Burke MC, Gold MR, Knight BP, Barr CS, Theuns DA, Boersma
LV, Knops RE, et al. Safety and efficacy of the totally subcutaneous
implantable defibrillator. J Am Coll Cardiol 2015; 65:16051615.

November 2015

1371